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Sclerosing Cholangitis

Virginia Mason is an international center for treating patients with biliary tract disorders, including primary sclerosing cholangitis. PSC is a chronic, progressive disease that causes damage to and blockage of the bile ducts in the liver. The term "sclerosing" means narrowing and "cholangitis" means infection of the bile ducts. While the connection is not clearly understood, about 75 percent of individuals with a type of inflammatory bowel disease (IBD) called ulcerative colitis develops PSC. It also may develop after a bacterial or viral infection or from an immune disorder.

The disease occurs more commonly in early middle age, in more men than in women, and in individuals with a family history of PSC. Unfortunately PSC damages the liver to such an extent that a liver transplant is the only cure. Progression from first diagnosis to a liver transplant ranges between 10 to 12 years. For more information or to schedule an appointment, call (206) 223-2319.

Symptoms of Primary Sclerosing Cholangitis

Disorders of the bile duct oftentimes have the same symptoms because most of them block the biliary ducts from releasing bile or digestive enzymes into the small intestine. Symptoms of primary sclerosing cholangitis may occur over time as scar tissue slowly develops.

Common symptoms and signs of primary sclerosing cholangitis include:

Abdominal pain on the right side of the body (where the liver and gallbladder are located)

Itching

Yellow skin or eyes (from the build up of bilirubin, a waste product)

Fatigue

Weight loss

Fever

Night sweats

Loss of appetite

Light-colored stools

Serious Complications

Over time, progressive primary sclerosing cholangitis can lead to scarring of the bile ducts (strictures) and of the liver (cirrhosis). Cirrhosis can lead to liver failure and the need for a liver transplant. Individuals with PSC also are at a higher risk of developing bile duct cancer and gallbladder cancer (cholangiocarcinoma), and individuals with both PSC and inflammatory bowel disease are at a higher risk of developing colon cancer. The presence of any symptoms shown above should be brought to the attention of a physician without delay.

Diagnosing Primary Sclerosing Cholangitis

Your gastroenterologist may suspect that you have a disorder of the biliary tract based on your medical history, your signs and symptoms, and the results of blood tests that may show high levels of bilirubin, a waste product in blood from the normal breakdown of red blood cells.

A definitive diagnosis is made through a procedure called ERCP, for endoscopic retrograde cholangiopancreatography, a procedure that allows your gastroenterologist to view the biliary and pancreatic ducts.

Additional tests and procedures for diagnosing primary sclerosing cholangitis include the following:

BLOOD TESTSIn addition to a bilirubin test, your blood may be tested for the presence of elevated white blood cells used by the body to fight infection, and for abnormal levels of pancreatic and liver enzymes. Other blood tests may be performed to rule out liver disorders such as cirrhosis or cancer.

ABDOMINAL ULTRASOUNDThis non-invasive procedure uses sound waves rather than x-rays to produce images. The images can reveal a narrowing within the common bile duct. During this procedure, an ultrasound probe is passed over the abdomen and images are sent to a computer monitor. Abdominal ultrasound is commonly used in women who are pregnant.

ABDOMINAL CT SCAN OR MRI A CT scan or MRI of the abdomen can identify narrowing within the biliary tract. Both scans are noninvasive procedures, during which the bile duct images are shown on a computer monitor.

ERCPEndoscopic retrograde cholangiopancreatography, or ERCP, is a specialized endoscopic technique used to study the ducts of the gallbladder, pancreas and liver, and has the added benefit of being a therapeutic tool. ERCP has been in use for more than 30 years, and is considered the standard modality for diagnosing and treating disorders of the biliary tract.

During this procedure, and after first receiving a mild sedative and an anesthetic to numb the throat, an endoscope containing a miniature camera is passed down your esophagus and into the biliary tract. When your gastroenterologist sees the biliary and pancreatic ducts, he or she then passes a catheter (a narrow plastic tube) containing a contrast dye through the endoscope. The dye is injected into the pancreatic and biliary ducts and x-rays are taken that are viewed on a computer monitor. The procedure takes 60 to 90 minutes and is performed in the Endoscopy Suite within Virginia Mason's Section of Gastroenterology and Hepatology.

Your gastroenterologist can treat a bile duct disorder at the same time it is being diagnosed by passing miniaturized instruments through the ERCP. Special preparations are required for this endoscopic procedure. Please see the ERCP prep planner for more information.

ERCP WITH ENDOSCOPIC ULTRASOUNDIncreasingly, gastroenterologists at Virginia Mason are using endoscopic ultrasound (EUS) in place of X-rays for better viewing of the bile and pancreatic ducts. During this procedure, an ultrasound probe is passed through the ERCP, which sends images to a computer monitor. Gastroenterologists can then treat disorders of the bile duct with miniaturized instruments passed through the ERCP.

MRCPMagnetic resonance cholangiopancreatography is newer technology being employed at Virginia Mason. This noninvasive diagnostic procedure is performed in Radiology using MRI technology (magnets and radio waves) to produce computer images of the bile ducts. A contrast dye is injected first through the skin near the gallbladder to enhance the images. Patients are not required to undergo endoscopy preparation and they do not undergo sedation. MRCP is being used primarily in patients who may have failed or who are not good candidates for ERCP, in those who do not want to undergo an endoscopic procedure, and in individuals considered to be at low risk of having a pancreatic or bile duct disorder. While ERCP allows for therapeutic options with cholangioscopy, MRCP is a diagnostic tool only.

Virginia Mason also is involved in national clinical trials to determine the accuracy of MRCP in diagnosing disorders of the biliary tract.

LIVER BIOPSYA sample of liver tissue may be taken to rule out cirrhosis or cancer. The sample can be taken at the time of diagnosis through the ERCP or with a thin needle injected through the skin (percutaneously) and into the liver.

Treating Primary Sclerosing Cholangitis

Therapy is given first to treat symptoms caused by this progressive disease. Itching, for example, a common symptom in biliary tract disorders, can be treated with antihistamines. If an infection is present, it will be treated with antibiotics first before endoscopic or surgical therapy is initiated. Vitamin deficiencies, from the lack of bile aiding digestion in the small intestine, can be treated with supplements.

ENDOSCOPIC THERAPYBile duct scarring and narrowing from PSC is commonly treated by placing a small stent (a hollow tube) within the bile duct to keep it open. This procedure can be performed at the time of diagnosis with miniaturized surgical instruments inserted through the ERCP.

If a narrowing or stricture completely blocks the bile duct - and if it is amenable to dilation - it can be widened first with a procedure called balloon dilation. A balloon on the tip of a catheter is passed through the ERCP and is inflated at the site of the narrowing, enlarging the blocked duct. A stent, also passed through the ERCP, is then placed within the duct and opened.

Pic of balloon dilation, widening the bile duct, and placing and opening a stent.

PERCUTANEOUS THERAPYWhen an ERCP procedure is not the best option for the patient, a percutaneous (through the skin) procedure to dilate a blocked bile duct and place a stent can be done instead.

SURGICAL THERAPYSurgical treatment is recommended when endoscopic therapy is not successful or if the bile duct stricture cannot be treated by endoscopic or percutaneous means. During surgery, the stricture is removed and the gallbladder or liver is connected to the small intestine. This open surgical procedure is performed in the operating room under a general anesthetic.

However, for most patients with primary sclerosing cholangitis, the disease damages the liver to such an extent that a liver transplant is the only viable option over time. A liver transplant is considered a cure for the disease. But the disease occurring in the new liver cannot be ruled out.